MCID: OSC001
MIFTS: 31

Oeis Complex

Categories: Nephrological diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Oeis Complex

MalaCards integrated aliases for Oeis Complex:

Name: Oeis Complex 57 59
Omphalocele-Cloacal Exstrophy-Imperforate Anus-Spinal Defect Syndrome 59
Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects 57
Omphalocele Exstrophy Imperforate Anus 73
Cloacal Exstrophy 59

Characteristics:

Orphanet epidemiological data:

59
cloacal exstrophy
Inheritance: Multigenic/multifactorial; Age of onset: Infancy,Neonatal; Age of death: normal life expectancy;

OMIM:

57
Miscellaneous:
majority of cases are sporadic
oeis is an acronym for omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects

Inheritance:
isolated cases


HPO:

32
oeis complex:
Inheritance sporadic


Classifications:



Summaries for Oeis Complex

MalaCards based summary : Oeis Complex, also known as omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome, is related to omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex and cloacal exstrophy. An important gene associated with Oeis Complex is MNX1 (Motor Neuron And Pancreas Homeobox 1). Affiliated tissues include kidney, colon and uterus, and related phenotypes are anal atresia and talipes equinovarus

Wikipedia : 76 Cloacal exstrophy (EC) is a severe birth defect wherein much of the abdominal organs (the bladder and... more...

Description from OMIM: 258040

Related Diseases for Oeis Complex

Diseases related to Oeis Complex via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 13)
# Related Disease Score Top Affiliating Genes
1 omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex 11.9
2 cloacal exstrophy 11.4
3 omphalocele 10.4
4 anus, imperforate 10.4
5 pentalogy of cantrell 10.0
6 nail-patella syndrome 9.8
7 neural tube defects 9.8
8 exstrophy of bladder 9.8
9 tethered spinal cord syndrome 9.8
10 lumbosacral lipoma 9.8
11 double discordia 9.8
12 limb-body wall complex 9.8
13 transposition of the great arteries 9.8

Graphical network of the top 20 diseases related to Oeis Complex:



Diseases related to Oeis Complex

Symptoms & Phenotypes for Oeis Complex

Symptoms via clinical synopsis from OMIM:

57
Neurologic Central Nervous System:
hydrocephalus
tethered cord
meningomyelocele
lipomyelocystoceles
chiari malformation

Abdomen Gastrointestinal:
anal atresia
intestinal malrotation
rectovaginal fistula
anteriorly placed anus
duplicated colon
more
Skeletal Spine:
hemivertebrae
absent sacrum
sacral segmentation defects

Genitourinary External Genitalia Female:
ambiguous genitalia
hypoplastic labia
widely separated labioscrotal folds

Abdomen External Features:
omphalocele

Chest Ribs Sternum Clavicles And Scapulae:
11 pairs of ribs

Genitourinary Internal Genitalia Female:
bifid uterus

GenitourinaryInternal GenitaliaMale:
cryptorchidism

Genitourinary Kidneys:
pelvic kidney
hydronephrosis
renal agenesis

Genitourinary External Genitalia Male:
ambiguous genitalia
epispadias
small penis
abnormal or absent scrotum

Genitourinary Ureters:
hydroureter
duplicated collecting system

Skeletal Pelvis:
congenital hip dislocation
symphysis pubis diastasis

Genitourinary Bladder:
cloacal exstrophy
vesicovaginal fistula

Skeletal Feet:
club feet


Clinical features from OMIM:

258040

Human phenotypes related to Oeis Complex:

59 32 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 anal atresia 59 32 Frequent (79-30%) HP:0002023
2 talipes equinovarus 59 32 Frequent (79-30%) HP:0001762
3 intestinal malrotation 59 32 Frequent (79-30%) HP:0002566
4 hemivertebrae 59 32 Frequent (79-30%) HP:0002937
5 hydroureter 59 32 Occasional (29-5%) HP:0000072
6 omphalocele 59 32 Very frequent (99-80%) HP:0001539
7 myelomeningocele 59 32 Frequent (79-30%) HP:0002475
8 bladder exstrophy 59 32 Very frequent (99-80%) HP:0002836
9 cloacal exstrophy 59 32 Very frequent (99-80%) HP:0010475
10 hydrocephalus 32 HP:0000238
11 ectopic kidney 59 Occasional (29-5%)
12 cryptorchidism 32 HP:0000028
13 horseshoe kidney 59 Occasional (29-5%)
14 renal hypoplasia/aplasia 59 Occasional (29-5%)
15 hip dislocation 59 Frequent (79-30%)
16 arnold-chiari malformation 32 HP:0002308
17 vesicoureteral reflux 59 Very frequent (99-80%)
18 spina bifida 59 Frequent (79-30%)
19 hypoplasia of penis 59 Very frequent (99-80%)
20 pelvic kidney 32 HP:0000125
21 rectovaginal fistula 32 HP:0000143
22 anteriorly placed anus 32 HP:0001545
23 ureteropelvic junction obstruction 59 Occasional (29-5%)
24 hydronephrosis 32 HP:0000126
25 epispadias 32 HP:0000039
26 ambiguous genitalia, male 32 HP:0000033
27 micropenis 32 HP:0000054
28 abnormality of tibia morphology 59 Frequent (79-30%)
29 renal agenesis 32 HP:0000104
30 congenital hip dislocation 32 HP:0001374
31 absence of the sacrum 32 HP:0010305
32 11 pairs of ribs 32 HP:0000878
33 ureterocele 59 Occasional (29-5%)
34 duplicated collecting system 32 HP:0000081
35 abnormality of the fibula 59 Frequent (79-30%)
36 labial hypoplasia 32 HP:0000066
37 intestinal duplication 59 Frequent (79-30%)
38 ambiguous genitalia, female 32 HP:0000061
39 abnormality of the fallopian tube 59 Occasional (29-5%)
40 abnormality of the clitoris 59 Very frequent (99-80%)
41 duplicated colon 32 HP:0005223
42 absent foot 59 Frequent (79-30%)
43 bifid uterus 32 HP:0000136
44 tethered cord 32 HP:0002144
45 vesicovaginal fistula 32 HP:0001586
46 absent scrotum 32 HP:0008707
47 sacral segmentation defect 32 HP:0008490

Drugs & Therapeutics for Oeis Complex

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prospective Cohort of Transitional Urology Patients Recruiting NCT03061084

Search NIH Clinical Center for Oeis Complex

Genetic Tests for Oeis Complex

Anatomical Context for Oeis Complex

MalaCards organs/tissues related to Oeis Complex:

41
Kidney, Colon, Uterus

Publications for Oeis Complex

Articles related to Oeis Complex:

(show all 32)
# Title Authors Year
1
A rare case of OEIS complex - newer approach to diagnosis of exstrophy bladder by color doppler and its differentiation from simple omphalocele. ( 29379239 )
2017
2
Discordant Anomalies with Combined Features of Pentalogy of Cantrell and OEIS Complex: A Case Report in Monochorionic Twins. ( 28617177 )
2017
3
Penile duplication as genital abnormality in OEIS complex: a fetal autopsy case report. ( 26513516 )
2016
4
Multimodality Renal Failure in a Patient with OEIS Complex. ( 26495176 )
2015
5
Fetal and neonatal presentation of OEIS complex. ( 26520699 )
2015
6
Early prenatal diagnosis of the OEIS complex with different appearance in early compared with late pregnancy before spontaneous rupture of the cloacal membrane. ( 24623061 )
2014
7
Re: Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 24703152 )
2014
8
Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex). ( 25400352 )
2014
9
Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 22640865 )
2013
10
OEIS complex. ( 24839537 )
2013
11
Prenatal findings of omphalocele-exstrophy of the bladder-imperforate anus-spinal defects (OEIS) complex. ( 22925220 )
2012
12
Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. ( 21453858 )
2011
13
Comprehensive genetic analysis of OEIS complex reveals no evidence for a recurrent microdeletion or duplication. ( 21204209 )
2011
14
Prenatal diagnosis of OEIS (omphalocele, bladder exstrophy, imperforate anus, clubfeet) variant associated with increased nuchal translucency and OEIS complex with ambiguous genitalia associated with corrected transposition of the great arteries: case series and review of the literature. ( 21475965 )
2011
15
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in a child with nail-patella syndrome. ( 20880310 )
2010
16
OEIS complex associated with chromosome 1p36 deletion: a case report and review. ( 20101692 )
2010
17
Supernumerary kidney in a child with OEIS complex. ( 19371928 )
2009
18
Discordant omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex and cardiac malformations in monochorionic twins. ( 19842883 )
2009
19
Double lumbosacral lipomas of the dorsal and filar types associated with OEIS complex: case report. ( 19855150 )
2009
20
Neurosurgical management of occult spinal dysraphism associated with OEIS complex. ( 17962955 )
2008
21
Syndromes and disorders associated with omphalocele (II): OEIS complex and Pentalogy of Cantrell. ( 17638617 )
2007
22
Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review. ( 17702047 )
2007
23
OEIS complex with major cardiac malformation: a case report. ( 17883075 )
2007
24
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex associated with increased nuchal translucency. ( 12858313 )
2003
25
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases. ( 11251992 )
2001
26
OEIS complex--a population study. ( 10797425 )
2000
27
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins. ( 10213043 )
1999
28
Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects. ( 9259943 )
1997
29
Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam. ( 7711456 )
1995
30
The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. ( 1433234 )
1992
31
OEIS complex (cloacal exstrophy): prenatal diagnosis in the second trimester. ( 2456564 )
1988
32
The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). ( 728566 )
1978

Variations for Oeis Complex

Expression for Oeis Complex

Search GEO for disease gene expression data for Oeis Complex.

Pathways for Oeis Complex

GO Terms for Oeis Complex

Sources for Oeis Complex

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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